Abstract
Purpose. To report a case of unilateral frosted branch angiitis (FBA) resembling central retinal vein occlusion associated with Familial Mediterranean Fever (FMF). Case Report. A 32-year-old woman presented with progressive, painless vision loss in her left eye lasting for 2 days. She was clinically diagnosed with FMF 2 months ago. The best-corrected visual acuity (BCVA) was 20/20 in her right eye and there was light perception in the left. Ophthalmologic examination revealed severe retinal vasculitis showing clinical features of FBA in the left eye. 64 mg/day oral methylprednisolone was started. A significant improvement in retinal vasculitis was observed in two weeks. However, BCVA did not increase significantly due to subhyaloid premacular hemorrhage. Argon laser posterior hyaloidotomy was performed. One week after hyaloidotomy, visual acuity improved to 20/20 and intravitreal hemorrhage disappeared. Four months after the first attack, FBA recurred. Oral methylprednisolone dosage was increased to 64 mg/day and combined with azathioprine 150 mg. At the end of 12-month follow-up, the BCVA was 20/25 and development of epiretinal membrane was observed in the left eye. Conclusions. Frosted branch angiitis may occur with gene abnormalities as an underlying condition. Our case showed that FMF might be a causative disease.
Highlights
Frosted branch angiitis (FBA) is a rare vascular disorder, first reported by Ito et al in 1976 in a six-year-old child [1]
FBA is characterized by acute visual loss, severe vascular inflammation, continuous sheathing of vascular structures, and retinal hemorrhages
Other autoinflammatory diseases have a much higher incidence of ocular disease than Familial Mediterranean Fever (FMF) which was reported as a secondary cause of ophthalmic involvement in a few cases [2]
Summary
Frosted branch angiitis (FBA) is a rare vascular disorder, first reported by Ito et al in 1976 in a six-year-old child [1]. FBA is characterized by acute visual loss, severe vascular inflammation, continuous sheathing of vascular structures, and retinal hemorrhages. As Ito et al described, FBA usually affects young and healthy persons [1]. Several unusual FBA cases associated with ophthalmic and systemic diseases were reported. Other autoinflammatory diseases have a much higher incidence of ocular disease than Familial Mediterranean Fever (FMF) which was reported as a secondary cause of ophthalmic involvement in a few cases [2]. We present an unusual and rare case of FBA secondary to FMF resembling central retinal vein occlusion
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